We describe a case of the juvenile form of Pompe's disease that presented as primary alveolar hypoventilation due to respiratory muscle involvement. This 17-year-old girl had been asymptomatic until this admission, although she had a delayed puberty. Arterial blood gas analysis, pulmonary function test as well as physical findings were compatible with chronic alveolar hypoventilation syndrome. Since she had lower extremity muscle weakness and pseudomyotonic discharge on electromyography a muscle biopsy was done, which revealed glycogen storage disease. The patient was managed successfully with nasal intermittent positive pressure ventilation.
Adolescent ; Chronic Disease ; Female ; Glycogen Storage Disease Type II/*complications/pathology ; Humans ; Hypoventilation/*etiology/therapy ; Intermittent Positive-Pressure Ventilation ; Muscles/pathology ; Pulmonary Alveoli

The purpose of this study was to evaluate the effect of the nutrition education on preschool children's nutrition knowledge and behaviors. The study subjects were preschool children attending J kindergarten located in Wonju. Each of the control group and the education group consisted of 49 children and the education group was exposed to nutrition education while the control group did not. The nutrition education intervention lasted 9 weeks weekly basis. The t-test for the homogeneity of each group showed no meaningful difference at the beginning point of nutrition education. After nutrition education a test of nutrition knowledge using picture-questionnaire was carried out by the researcher for the subjects of education group and control group and the caretakers of the education group were offered self-administered questionnaire on the changes in children's dietary behaviors. The results of the study were as follows: 1. Nutrition education intervention showed a meaningful difference in children's understanding nutrition; the kinds and function of food, the relationship between food and body, the function of nutrients and enhanced their idea on hygiene compared to the control group (p < p.005). 2. However, nutrition education intervention made no statistically meaningful difference in children's dietary behaviors such as having breakfast, keeping balanced diet, washing hands before meals, saying "thanks" before and after meals, cleaning the table after meals, brushing teeth after meals and so on. Therefore, we found that it is hard to expect children to correct their dietary behaviors in such a short period of 9 weeks and nutrition education for preschool children should be offered to caretakers at the same time.
Breakfast ; Child ; Child, Preschool ; Diet ; Hand ; Humans ; Hygiene ; Meals ; Surveys and Questionnaires ; Tooth

PURPOSE: Chronic constipation is not uncommon even in children. Recently biofeedback treatment (BT) is considered as a useful modality in the treatment of various types of constipation. The aim of this study was to evaulate the effectiveness of BT in children with chronic constipation. METHODS: We reviewed 24 cases of childhood constipation (17 boys, 7 girls) who visited the Jinju Hakmoon colorectal surgery clinic and GNUH1 between April, 2001 and September, 2002. Balloon-mediated anorectal manometry system was used for BT. The effects of therapy was assessed by bowel movement, defecation time, fecal soiling and balloon evacuation test in the early course of therapy. The long term follow up survey was done by telephone interview at January 2003 and the effects of therapy was assessed by bowel movement, defecation time, fecal soiling and parents' satisfaction. RESULTS: Patients received the BT for the three times on average, and as the result of the therapy, 20 patients experienced disappearance of symptoms (87%) or at least improvement of symptoms. In terms of manometric values, the mean resting pressure was 113.4 mmHg before the BT, but decreased to 86.3 mmHg after the BT (P=0.0012). Mean pushing pressure dropped to 85.98 mmHg from 130.45 mmHg (P=<0.0001). Mean sensitivity decreased from 55 ml to 35 ml (P=0.0053). Mean compliance decreased from 20.28 H2O/cm to 4.21 H2O/cm (P=0.0015). 13 patients (76%) showed disappearance or improvement of symptoms in the long term follow up. CONCLUSIONS: The Balloon mediated biofeedback therapy is effective in children with chronic constipation. It is safe, easy to administer and also improve symptoms and objective parameters of anorectal function. Balloon mediated biofeedback therapy could be the first-line therapy in child patient with chronic constipation who failed conservative management.
Biofeedback, Psychology* ; Child* ; Colorectal Surgery ; Compliance ; Constipation* ; Defecation ; Follow-Up Studies ; Gyeongsangnam-do ; Humans ; Interviews as Topic ; Manometry ; Soil

To evaluate the prognostic significance of ERD5 in gastric cancer, the frequency of their expression was examined by the immunohistochemical method in 70 cases of gastric cancer with alcohol-fixed, paraffin embedded specimens which were obtained surgically at the department of Surgery, Gyeongsang National University Hospital from October, 1990 to May, 1993. Correlations between ERD5 positivity and clinicopathological parameters were analysed with Student's t-test and Chi-square test, and the survival rate according to whether the ERD5 was present or not was evaluated with Kaplan-Meier analysis model. Of the 70 patients, 40 cases showed a positive rate for estrogen receptor and 27 cases(67.5%) were male and 15 cases(50%) were female. There was no significant difference between the ERD5 positive rate and sex distribution. In age distribution, the ERD5 positive rate was most high(67%) in the 5th decade, but it was not significant statistically. There was no significant difference between the ERD5 positive rate and the WHO pathologic classification. In Lauren's classification, the intestinal type of gastric cancer had a higher positive rate(69%) than diffuse type(50%). In Ming's classification, the expanding type(82%) of gastric cancer had a higher positive rate than the infiltrative type(50%). A statistically significant difference was found between the expanding type and infiltrative type(P<0.05). There were no significant differences between the ERD5 positive rate and the TNM staging or the degree of differentiation of cancer cells. There was no significant difference between the ERD5 positive rate and the serum CEA level. Of the 70 patients, the overall 5-years survival rate was 37 % and 5-years survival rate of positive ERD5 cases had a higher rate (38.4%) than negative cases (35.1%) but there was no statistically significant difference. In conclusion, the ERD5 positive rate was significantly high in the patient with expanding type of gastric cancer in Ming's classification and intestinal type of gastric cancer in Lauren's classification. But, there was no significant difference in the survival rates between the ERD5 positive group and the ERD5 negative group.
Age Distribution ; Classification ; Estrogens* ; Female ; Humans ; Kaplan-Meier Estimate ; Male ; Neoplasm Staging ; Paraffin ; Sex Distribution ; Stomach Neoplasms* ; Survival Rate

BACKGROUND: Organochloride pesticides are prohibited around the world due to their toxicity and environmental persistence. However, lindane, one organochloride pesticide, is still widely used to treat head lice and scabies in Korea. OBJECTIVE: For evaluation of the effect of lindane usage and blood lindane concentration, we conduct an evaluation of blood lindane concentrations among children who were treated for head lice. METHODS: The total study population of 80 was divided into rural and urban areas. Age, sex, parent's farming, sea food consumption and head lice treatment information were gathered using a structured questionnaire. Blood lindane concentrations were analyzed by a solid phase extraction(SPE) method. Quantitative analysis was done by GC/ECD. Non-parametric analysis was done for comparing blood lindane concentrations between the head lice treated group and the non treatment group. After a log transformation, regression analysis was done for controlling confounding variables. RESULTS: The median age of the study population was 11. The study population involved 38 (47.5%) male and 42 (52.5%) female children. Twenty (25.6%) children were infested by head lice. The blood concentration of lindane was higher in the head licetreatment group [N.D (N.D-78.9)] than in the non-treated group [N.D (N.D-43.4), P < 0.01]. The results were the same after regression analysis controlling for confounding variables. CONCLUSION: Lindane has toxicity to the endocrine system and nervous system and persists in the environment and in the human body. Therefore, careful usage of lindane shampoo is needed to treat head lice.
Child ; Confounding Factors (Epidemiology) ; Endocrine System ; Female ; Head ; Human Body ; Humans ; Lindane ; Male ; Nervous System ; Pediculus ; Pesticides ; Scabies ; Seafood ; Surveys and Questionnaires

BACKGROUND: Organochloride pesticides are prohibited around the world due to their toxicity and environmental persistence. However, lindane, one organochloride pesticide, is still widely used to treat head lice and scabies in Korea. OBJECTIVE: For evaluation of the effect of lindane usage and blood lindane concentration, we conduct an evaluation of blood lindane concentrations among children who were treated for head lice. METHODS: The total study population of 80 was divided into rural and urban areas. Age, sex, parent's farming, sea food consumption and head lice treatment information were gathered using a structured questionnaire. Blood lindane concentrations were analyzed by a solid phase extraction(SPE) method. Quantitative analysis was done by GC/ECD. Non-parametric analysis was done for comparing blood lindane concentrations between the head lice treated group and the non treatment group. After a log transformation, regression analysis was done for controlling confounding variables. RESULTS: The median age of the study population was 11. The study population involved 38 (47.5%) male and 42 (52.5%) female children. Twenty (25.6%) children were infested by head lice. The blood concentration of lindane was higher in the head licetreatment group [N.D (N.D-78.9)] than in the non-treated group [N.D (N.D-43.4), P < 0.01]. The results were the same after regression analysis controlling for confounding variables. CONCLUSION: Lindane has toxicity to the endocrine system and nervous system and persists in the environment and in the human body. Therefore, careful usage of lindane shampoo is needed to treat head lice.
Child ; Confounding Factors (Epidemiology) ; Endocrine System ; Female ; Head ; Human Body ; Humans ; Lindane ; Male ; Nervous System ; Pediculus ; Pesticides ; Scabies ; Seafood ; Surveys and Questionnaires

PURPOSE: Malnutrition in hospitalized children has an impact on growth, morbidity, and mortality. For this reason, the implementation of pediatric nutrition support team (PNST) has been suggested. On April 2017, in Severance Hospital, more PNST physician's departments participated in PNST, and a PNST physician assigning system was changed to be the same departments with the attending physician and PNST physician. This study performed a comparative analysis of the compliance and clinical outcomes after expanding the participation of the PNST physician's department. METHODS: Pediatric patients, who were referred to a PNST consultant, were divided into two groups: an unmatched group (different departments with the attending physician and PNST physician [154 patients, 233 consultations from May 2016 to October 2016]), and a matched group (same departments with the attending physician and PNST physician [169 patients, 302 consultations from May 2017 to October 2017]). The PNST compliance and clinical outcomes, such as the total delivered/required caloric and protein ratio, % ideal body weight (%IBW), serum total protein, and serum albumin, in the two groups were compared. RESULTS: The compliance was significantly higher in the matched group than the unmatched group (63.4% vs. 47.3%, P=0.005). Although there was no significant difference, the total delivered/required caloric and protein ratio, and %IBW in the matched group tended to increase. The serum total protein (0.7±0.7 g/dL vs. −0.4±1.3 g/dL, P=0.004) and serum albumin (0.5±0.5 g/dL vs. −0.1±0.6 g/dL, P=0.003) were significantly higher in the matched group. CONCLUSION After expanding the physician's departments in PNST, the compliance was significantly higher in the matched group and the clinical outcomes tended to better. The physician assigning system to be matched with the departments between the attending physician and the PNST physician may help increase the compliance of NST consultations, resulting in an improvement of the patient's clinical outcomes.

Background: During the coronavirus disease 2019 (COVID-19) pandemic, there was a shortage of medical resources and the need for proper treatment guidelines for brain tumor patients became more pressing. Thus, the Korean Society for Neuro-Oncology (KSNO), a multidisciplinary academic society, has undertaken efforts to develop a guideline that is tailored to the domestic situation and that can be used in similar crisis situations in the future. As part II of the guideline, this consensus survey is to suggest management options in specific clinical scenarios during the crisis period. Methods: The KSNO Guideline Working Group consisted of 22 multidisciplinary experts on neuro-oncology in Korea. In order to confirm a consensus reached by the experts, opinions on 5 specific clinical scenarios about the management of brain tumor patients during the crisis period were devised and asked. To build-up the consensus process, Delphi method was employed. Results: The summary of the final consensus from each scenario are as follows. For patients with newly diagnosed astrocytoma with isocitrate dehydrogenase (IDH)-mutant and oligodendroglioma with IDH-mutant/1p19q codeleted, observation was preferred for patients with low-risk, World Health Organization (WHO) grade 2, and Karnofsky Performance Scale (KPS) ≥60, while adjuvant radiotherapy alone was preferred for patients with high-risk, WHO grade 2, and KPS ≥60. For newly diagnosed patients with glioblastoma, the most preferred adjuvant treatment strategy after surgery was radiotherapy plus temozolomide except for patients aged ≥70 years with KPS of 60 and unmethylated MGMT promoters. In patients with symptomatic brain metastasis, the preferred treatment differed according to the number of brain metastasis and performance status. For patients with newly diagnosed atypical meningioma, adjuvant radiation was deferred in patients with older age, poor performance status, complete resection, or low mitotic count. Conclusion It is imperative that proper medical care for brain tumor patients be sustained and provided, even during the crisis period. The findings of this consensus survey will be a useful reference in determining appropriate treatment options for brain tumor patients in the specific clinical scenarios covered by the survey during the future crisis.

Background: During the coronavirus disease 2019 (COVID-19) pandemic, the need for appropriate treatment guidelines for patients with brain tumors was indispensable due to the lack and limitations of medical resources. Thus, the Korean Society for Neuro-Oncology (KSNO), a multidisciplinary academic society, has undertaken efforts to develop a guideline that is tailored to the domestic situation and that can be used in similar crisis situations in the future. Methods: The KSNO Guideline Working Group was composed of 22 multidisciplinary experts on neuro-oncology in Korea. In order to reach consensus among the experts, the Delphi method was used to build up the final recommendations. Results: All participating experts completed the series of surveys, and the results of final survey were used to draft the current consensus recommendations. Priority levels of surgery and radiotherapy during crises were proposed using appropriate time window-based criteria for management outcome. The highest priority for surgery is assigned to patients who are life-threatening or have a risk of significant impact on a patient’s prognosis unless immediate intervention is given within 24–48 hours. As for the radiotherapy, patients who are at risk of compromising their overall survival or neurological status within 4–6 weeks are assigned to the highest priority. Curative-intent chemotherapy has the highest priority, followed by neoadjuvant/adjuvant and palliative chemotherapy during a crisis period. Telemedicine should be actively considered as a management tool for brain tumor patients during the mass infection crises such as the COVID-19 pandemic. Conclusion It is crucial that adequate medical care for patients with brain tumors is maintained and provided, even during times of crisis. This guideline will serve as a valuable resource, assisting in the delivery of treatment to brain tumor patients in the event of any future crisis.

We report a rare case of lung disease caused by Mycobacterium lentiflavum in a previously healthy woman. A 54-year-old woman was referred to our hospital due to chronic cough and sputum. A computed tomography scan of the chest revealed bilateral bronchiectasis with bronchiolitis in the right middle lobe and the lingular division of the left upper lobe. Nontuberculous mycobacteria were isolated twice from three expectorated sputum specimens. All isolates were identified as M. lentiflavum by multilocus sequence analysis based on rpoB, hsp65, and 16S rRNA fragments. To the best of our knowledge, this is the first documented case of M. lentiflavum lung disease in an immunocompetent adult in Korea.
Adult ; Bronchiectasis ; Bronchiolitis ; Cough ; Female ; Humans ; Korea ; Lung ; Lung Diseases ; Multilocus Sequence Typing ; Mycobacterium ; Mycobacterium Infections, Nontuberculous ; Nontuberculous Mycobacteria ; Sputum ; Thorax